CT findings of glycogen storage disease I complicated with pancreatitis: A case report

Rationale: The incidence of glycogen storage disease type I (GSD I) in the overall population is 1/100,000.[1] Hyperlipidemia in patients with GSD I can induce pancreatitis. Three cases of GSD I complicated with pancreatitis have been reported.[2] Here, the computed tomography (CT) features of GSD I complicated with pancreatitis are reported for the first time Patient concerns: A 22-year-old female presents with growth retardation for 20 years and recurrent epigastric pain for 3 years. No abnormality in physical examination. Laboratory examination: GPT 81 U/L, GOT 111 U/L, DBIL 1.7 umol/L, TBIL 0.7 umol/L, Albumen 41.4 g/L, blood ammonia 54 umol/L, fasting blood glucose 3.02 mmol/L, G6PD 1829 U/L, lactic acid 7.9 mmol/L, triglyceride 18.79 mmol/L, TCH 9.46 mmol/L, uric acid 510 umol/L, urinary protein +++ (3.0) g/L. Diagnosis: The CT findings of the upper abdomen show that the liver is obviously enlarged, and the density of the liver is obviously uneven on plain scan. Unclear boundaries and increased blood vessels of the pancreas are found, especially in the head of the pancreas. The patient is diagnosed with GSD I complicated with pancreatitis. Interventions: The patient undergoes split liver transplantation and splenectomy under general anesthesia in our hospital. Outcomes: Upper abdominal CT is reexamined half a month and 2 and a half months after the operation. It is found that the transplanted liver is not enlarged and the density is not abnormal. The pancreas shrinks, its boundary is clear, and its blood vessels decrease, especially in the head of the pancreas Lessons: The density of the liver depends on the relative amount of glycogen and fat, which can be increased, normal, or decreased. Hyperlipidemia in patients with GSD I can induce pancreatitis.


Introduction
A case of glycogen storage disease I complicated with pancreatitis is reported. The computed tomography (CT) features are summarized as follows. The liver is enlarged. There are high-density glycogen storage areas and low-density fat infiltration areas in the plain CT scan of the liver. Unclear boundaries and increased blood vessels in the pancreas are found.
The CT findings of the upper abdomen show that the liver is obviously enlarged, the density of the liver is obviously uneven on plain scan, and the density of the right lobe of the liver is slightly lower than that of the left lobe of the liver. The CT value of the right lobe of the liver is between 12 and 69HU and the CT value of the left lobe of the liver is between 24 and 76HU ( Fig. 1). The liver is gradually enhanced. Unclear boundaries Medicine and increased blood vessels of the pancreas are found, especially in the head of the pancreas (Fig. 2).
The patient is diagnosed with glycogen storage disease type I (GSD I) complicated with pancreatitis, who has the indication for liver transplantation [3] and has no specific contraindications for liver transplantation. The patient undergoes split liver transplantation and splenectomy under general anesthesia in our hospital. A small amount of ascites is detected during the operation, and the liver is tough and enlarged in volume. Microscopically, the normal structure of the hepatic lobule is destroyed and a false lobule is formed. The hepatocytes are obviously enlarged and the staining becomes lighter. The hepatocytes are disorderly arranged and the hepatic sinusoids are compressed. Steatosis is seen in hepatocytes. A few inflammatory cells infiltrate the stroma. Fibrous tissue proliferates and forms fibrous septum (Fig. 3). Immunohistochemistry PAS (+) (Fig. 4).
Upper abdominal CT is reexamined half a month and 2 and a half months after the operation. It is found that the transplanted liver is not enlarged and the density is not abnormal. The pancreas shrinks, its boundary is clear, and its blood vessels decrease, especially in the head of the pancreas. Ischemic infarcted area without enhancement is seen in the head of the pancreas (Figs. 5 and 6).

Discussion
GSD I is an autosomal recessive inborn error of carbohydrate metabolism caused by defects of the glucose-6-phosphatase complex. [2] Deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestine results in the accumulation of glycogen in these organs. [2] As a result of inadequate glucose production, patients have severe fasting hypoglycemia with secondary Figure 1. A plain CT scan of the upper abdomen. The liver is obviously enlarged, its density is obviously uneven, and the mosaic-like appearance is seen. The density of the right lobe is slightly lower than that of the left lobe. CT = computed tomography.   . Enhanced CT scans of the upper abdomen half a month and 2 and a half months after the operation, respectively. The pancreas shrinks, its boundary is clear, and its blood vessels decrease, especially in the head of the pancreas. Ischemic infarcted area without enhancement is seen in the head of the pancreas (black arrow). CT = computed tomography. www.md-journal.com biochemical abnormalities: hyperlactacidemia, hyperuricemia, and hyperlipidemia. [2] The incidence of GSD I in the overall population is 1/100,000. [1] Hyperlipidemia in patients with GSD I can induce pancreatitis. Three cases of GSD I complicated with pancreatitis have been reported. [2] Here, the CT features of GSD I complicated with pancreatitis are reported for the first time.
Studies have shown that diffuse or focal high-density areas in the plain CT scan for the liver, are more glycogen deposition areas in the histological examination in patients with GSD I. If there is fat infiltration at the same time, it can completely or partially counteract the effect of glycogen on liver density. The density of the liver depends on the relative amount of glycogen and fat, which can be increased, normal, or decreased. [4]

Conclusion
Here, the CT features of GSD I complicated with pancreatitis are reported for the first time. The liver is enlarged. There are high-density glycogen storage areas and low-density fat infiltration areas in the plain CT scan of the liver. Unclear boundaries and increased blood vessels in the pancreas are found.